Cases reported "Sarcoidosis"

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1/12. Transesophageal echocardiographic abnormalities in a case of cardiac sarcoidosis.

    sarcoidosis is a granulomatous disease that may involve multiple organ systems, including the heart. Manifestations include atrial and ventricular arrhythmias, conduction abnormalities, congestive cardiac failure, pericarditis, and sudden death. Whereas cardiac involvement is a relatively common finding at autopsy, antemortem diagnosis is often missed because the clinical manifestations are nonspecific, and the sensitivity and specificity of investigations are low. We report a case of a 62-year-old woman who had clinically significant cardiac sarcoidosis associated with echocardiographic abnormalities that had not been reported previously in association with this condition.
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2/12. PET and PLAP in suspected testicular cancer relapse: beware sarcoidosis.

    A 31-year-old man previously treated with chemotherapy for metastatic testicular cancer presented with new mediastinal lymphadenopathy and peripheral lung opacities. Serum tumour markers were not elevated and a PET (positron emission tomography) scan revealed increased FDG (fluoro-deoxyglucose) uptake in the lungs and mediastinum consistent with testis cancer relapse. A biopsy of a mediastinal lymph node was performed and the pathology was that of sarcoidosis. immunohistochemistry however was positive for PLAP (placental alkaline phosphatase) and negative for EMA (epithelial membrane antigen). This immunohistochemical profile raised concerns that the observed pathology represented a sarcoid reaction to micro-metastatic testicular cancer relapse. We performed immunohistochemical pathology analysis on four known cases of sarcoidosis and found the same immunohistochemical-staining pattern. This case highlights the problem of specificity when interpreting the significance of PET scans and immunohistochemical analysis in this situation. sarcoidosis, a condition that has been associated with testicular cancer, should always be considered in the differential diagnosis.
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3/12. sarcoidosis or Sjogren syndrome? Clues to defining mimicry or coexistence in 59 cases.

    We present 5 new cases of coexisting sarcoidosis and Sjogren syndrome (SS) and review the literature for additional cases in order to analyze the clinical, immunologic, and histologic characteristics that may help physicians differentiate the mimicry of SS by sarcoidosis from a true coexistence of both autoimmune diseases. We considered the coexistence of sarcoidosis with SS to be when patients presented specific histologic patterns of both diseases, simultaneously or at different times.Fifty-nine patients were included in the analysis (54 identified in the literature search plus our 5 unpublished cases): 49 (83%) patients were female and 10 (17%) were male, with a mean age at diagnosis of 50 years. According to the histopathologic examination of the exocrine glands performed in 53 cases, we defined coexistence of sarcoidosis and SS in 28 cases, while in the remaining 25 patients, sarcoidosis mimicked SS. Clues to identifying when sarcoidosis coexists with SS were a higher prevalence of systemic manifestations (arthritis and uveitis) and positive immunologic parameters (antinuclear antibodies, rheumatoid factor, and anti-Ro/SS-A), as well as the existence of a focal sialadenitis (Chisholm-Mason score grades III-IV, with a CD4 lymphocytic infiltration) in the salivary gland biopsy. In patients first diagnosed with primary SS, the appearance of some clinical features such as hilar adenopathies, uveitis, or hypercalcemia leads to the diagnosis of coexisting sarcoidosis. A careful application of the new American-European consensus criteria had a sensitivity of 93% and a specificity of 92% in identifying when SS coexists with sarcoidosis.In conclusion, the association of sarcoidosis with SS leads to a true coexistence of both diseases in more than half the patients described in the literature, while in the remaining patients, sarcoidosis mimics SS. In light of these results, sarcoidosis should not be considered as an exclusion criterion for the diagnosis of SS, and in patients with a suspected overlap of the two diseases, application of the new American-European consensus criteria for diagnosis of SS should be mandatory.
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keywords = specificity
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4/12. Ghost lesions in patient with cerebral-isolated neurosarcoidosis. A case report.

    We present a case of isolated cerebral neurosarcoidosis with remitting nodular lesions resembling intraventricular neoplasms. The patient, admitted at our Department for surgical treatment of a magnetic resonance imaging (MRI) demonstrated fourth ventricle lesion, presented acute intracranial hypertension. A second MRI performed before the scheduled operation showed the disappearance of the fourth ventricle lesion and a tetra-ventricular hydrocephalus. The patient has been treated with a third-ventriculostomy, followed, after 15 days, by ventriculoperitoneal shunt. High doses of steroids have been administered. cerebrospinal fluid analysis has been conducted and a high concentration of ACE, specific marker of neurosarcoidosis, has been found. No biopsy was performed to avoid surgical complications. A systemic chemotherapy with azathioprine has been started, but the patient died six months later for worsening of the clinical conditions. The autopsy confirmed the diagnosis of neurosarcoidosis. This is the first MRI documented case of neurosarcoidosis with remitting lesions. diagnosis of isolated neurosarcoidosis is difficult and it is based on clinical and radiological exclusion of other entities. CSF examination is useful for diagnosis, for the reported specificity and sensibility of ACE. Medical treatment is based on corticosteroids and chemotherapic agents. Neurosurgical intervention can be related to treat hydrocephalus due to ependymal and arachnoidal involvement or to remove large lesions. In our opinion biopsy should be limited only to ACE negative patients and to those who do not respond to chemotherapy.
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5/12. "Dural tail sign": a specific MR sign for meningioma?

    Somewhat conflicting reports have appeared about the significance of linear meningeal thickening and enhancement adjacent to peripherally located cranial mass lesions on contrast enhanced magnetic resonance images. Some authors consider this finding nearly diagnostic of meningioma. In an attempt to determine the specificity of this so-called tail sign, particularly with respect to meningioma, we retrospectively reviewed 16 cases from institutional records. From our results, the tail sign appears to be highly suggestive but not specific for meningioma.
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6/12. Hodgkin's disease in sarcoidosis. A case report and a review of the literature.

    A patient with Hodgkin's disease preceded by sarcoidosis is presented and the difficulties introduced by the lack of specificity for the sarcoid-like granulomas to differentiate between the dual pathology are discussed. A review of the literature disclosed 9 similar case reports. It is suggested that the finding of non-caseating epithelioid cell granulomas in relatively old patients with lymphadenopathy, systemic symptoms and absolute neutrophil leucocytosis could be warning signals of the appearance of Hodgkin's disease.
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ranking = 1
keywords = specificity
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7/12. Gallium67 citrate scanning in patients with lacrimal gland and conjunctival sarcoidosis. A report on three cases.

    gallium citrate Ga67 scanning of the head and thorax of 3 patients with sarcoidosis is presented. Two patients had a progressive pulmonary sarcoidosis of recent onset and in one patient, with a chronic course of the disease, the lung changes had resolved. All 3 patients had a conjunctival sarcoid change confirmed by biopsy and a lacrimal gland affection suggested by clinical examination. A highly increased Ga67 uptake in the lacrimal and parotid glands as well as in the nasopharynx was found in all patients, and an increased lung uptake was found in the newly detected cases. It is concluded that gallium scanning is a sensitive method for detecting minute ophthalmic changes in sarcoidosis. It also reveals chronic localized ocular affection in cases in which the lung changes are no longer detectable. The mechanism and specificity of gallium uptake in sarcoidosis is also discussed.
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ranking = 1
keywords = specificity
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8/12. Intrathecal production of oligoclonal IgM and IgG in CNS sarcoidosis.

    A longitudinal study of multiple paired CSF and serum specimens from a patient with CNS sarcoidosis revealed high CSF IgM and IgG indices as well as oligoclonal IgM and IgG bands in CSF reflecting intrathecal IgM and IgG production. The antibody specificity of intrathecally-produced IgM and IgG remained undefined despite analysis for antibodies against mycobacterium tuberculosis and Kveim suspension. Steroid treatment induced rapid and complete clinical remission, and also decrease of CSF IgM and IgG antibodies, while oligoclonal IgM and IgG persisted in CSF. Repeated determinations of these CSF variables together with cell count and CSF/serum albumin ratio as a variable of blood-brain barrier function, might be useful in assessing effect of therapy in CNS sarcoidosis.
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ranking = 1
keywords = specificity
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9/12. sarcoidosis associated with autoimmune haemolytic anaemia and paroxysmal nocturnal haemoglobinuria red cell abnormality.

    A 29-year-old male with sarcoidosis autoimmune haemolytic anaemia and paroxysmal nocturnal haemoglobinuria is described. Throughout his illness the chest films showed fibrosis in the right hilar region and he had had several pneumonias in the right lung. He had had massive splenomegaly and a splenectomy was performed. He was treated with prednisone and cyclophosphamide. Because his blood group was initially confused, several incompatible blood transfusions were given. Two types of antibody were detected: an autoantibody with "s" specificity and an alloantibody with Rh "D" specificity. Other interesting features in this case revealed at autopsy were a viral pneumonia and toxoplasma gondii infection of the brain. As far as we know, this is the first reported patient with this unusual association.
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ranking = 2
keywords = specificity
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10/12. Prolonged course of illness in a child with malignant lymphoma mimicking sarcoidosis.

    We describe a case of malignant lymphoma mimicking the rheumatic presentation of sarcoidosis in an adolescent with a 3-year history of febrile illness. Final diagnosis was established by tissue biopsy after multiple studies failed to provide histological evidence of granulomas consistent with sarcoidosis. We discuss the limited diagnostic specificity of serum angiotensin converting enzyme in sarcoidosis and emphasize the need for aggressive diagnostic evaluation of a patient whose clinical presentation is not fully explained by a known rheumatologic illness.
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ranking = 1
keywords = specificity
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